Journal of Clinical Apheresis 00:00–00 (2015)
Safety and Efficacy of Blood Exchange Transfusion for Priapism Complicating Sickle Cell Disease Samir K. Ballas* and David Lyon† Cardeza Foundation for Hematologic Research, Department of Medicine, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania Background: Priapism is unwanted painful penile erection that affects about 36% of boys and men with sickle cell disease (SCD) most of whom have sickle cell anemia. Clinically, priapism could be stuttering, minor, or major. The first two types are mild, last < 4 h, are usually treated at home, have good prognosis with normal sexual function. The major type of priapism lasts >4 h, associated with severe pain, requires hospitalization; often does not respond to medical treatment and may require shunt surgery. Untreated major priapism and surgical intervention often cause impotence. In this study, we report our 15-year experience in treating adult patients with SCD and major priapism with blood exchange transfusion after being refractory to other medical therapies. Methods: Adult male African Americans patients with SCD and major priapism were enrolled in this study and followed for 15 years. A Haemonitics V-50 machine was initially used for whole blood exchange and was later replaced with Cobe Spectra machine for RBC exchange. Results: We used 239 blood exchanges requiring 1,136 RBC units. We maintained a post-exchange hemoglobin level of about 10 g/dL and hemoglobin S level < 30%. None of the patients had any neurological complications such as headache, seizures, neurological deficits, or obtundation post-exchange. Conclusion: Together, the data indicate that blood exchange transfusion for the treatment of patients with SCD C 2015 Wiley Periodicals, Inc. and major priapism is efficacious and safe. J. Clin. Apheresis 00:000–000, 2015. V Key words: impotence; shunt surgery; neurological complications; safety
INTRODUCTION
Priapism is unwanted and sustained painful erection lasting 4 or more hours. It is a common complication of sickle cell disease (SCD) affecting 89% of males with at least one episode of priapism by the time they are 20 years old [1]. It is most common in patients with SS, who account for approximately 80–90% of reported cases [2–5]. However, it does occur in all forms of SCD including Hb SC disease, sickle thalassemia, and in sickle trait [2,6]. Clinically, priapism may be stuttering, minor, or major. Stuttering priapism is the occurrence of short, repetitive, and reversible painful episodes with detumescence occurring within 30% in SCD increases whole blood viscosity significantly compared to normal. From Ballas SK. Sickle Cell Pain, 2nd Ed. Washington, DC: IASP; 2014. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.] TABLE IV.
Parameter Hemoglobin
Hematocrit
Reported Hematologic Values before and after Red Blood Cell Exchange Transfusion Pre-exchange
Post-exchange
(1) 6.3
(1) 13.5
(6) 7.3 6 1.4
(6) 12.1 6 1.1
(166) 7.6 6 1.8 N/A
(162) 9.1 6 1.5a N/A
N/A
N/A
(161) 22.8 6 5.5
(167) 26.8 6 4.8
Reference Siegel et al., 1993 [14] Rackoff et al., 1992 [13] This report Siegel et al., 1993 [14] Rackoff et al., 1992 [13] This report
N/A: not available. a P < 0.001 compared to the values in the first two rows.
viscosity. This dual cause of blood viscosity was reported in a patient with SS and acute chest syndromes (ACS) where blood exchange transfusion was not effective in resolving the ACS despite the reduction of Hb S to < 30% [24]. Plasmapheresis following blood exchange transfusion in this patient was effective in resolving the ACS [24].
1. US Department of Health and Human Services. Expert Panel Report. Evidence-Based Management of Sickle Cell Disease. Bethesda, MD: National Institutes of Health, National Heart, Lung and Blood Institute; 2014. 2. Adeyoju AB, Olujohungbe AB, Morris J, Yardumian A, Bareford D, Akenova A, Akinyanju O, Cinkotai K, O’Reilly PH. Priapism in sickle-cell disease; incidence, risk factors and complications—an international multicentre study. BJU Int 2002;90:898–902. 3. Powars DR, Johnson CS. Priapism. Hematol Oncol Clin North Am 1996;10:1363–1372. 4. Rogers ZR. Priapism in sickle cell disease. Hematol Oncol Clin North Am 2005;19:917–928, viii. 5. Sharpsteen JR Jr., Powars D, Johnson C, Rogers ZR, Williams WD, Posch RJ. Multisystem damage associated with tricorporal priapism in sickle cell disease. Am J Med. 1993;94:289–295. 6. Okpala I, Westerdale N, Jegede T, Cheung B. Etilefrine for the prevention of priapism in adult sickle cell disease. Br J Haematol 2002;118:918–921. 7. Emond AM, Holman R, Hayes RJ, Serjeant GR. Priapism and impotence in homozygous sickle cell disease. Arch Intern Med 1980;140:1434–1437. 8. Serjeant GR, de Ceulaer K, Maude GH. Stilboestrol and stuttering priapism in homozygous sickle-cell disease. Lancet 1985;2: 1274–1276. 9. Mantadakis E, Ewalt DH, Cavender JD, Rogers ZR, Buchanan GR. Outpatient penile aspiration and epinephrine irrigation for young patients with sickle cell anemia and prolonged priapism. Blood 2000;95:78–82. [PMID: 10607688] 10. Baron M, Leiter E. The management of priapism in sickle cell anemia. J Urol 1978;119:610–611. 11. Seeler RA. Intensive transfusion therapy for priapism in boys with sickle cell anemia. J Urol 1973;110:360–363. 12. Talacki CA, Ballas SK. Modified method of exchange transfusion in sickle cell disease. J Clin Apher 1990;5:183–187. [PMID: 2228996] 13. Rackoff WR, Ohene-Frempong K, Month S, Scott JP, Neahring B, Cohen AR. Neurologic events after partial exchange transfusion for priapism in sickle cell disease. J Pediatr 1992;120:882–885 14. Siegel JF, Rich MA, Brock WA. Association of sickle cell disease, priapism, exchange transfusion and neurological events: ASPEN syndrome. J Urol 1993;150(5 Part 1):1480–1482. 15. Betke K, Marti HR, Schlicht I. Estimation of small percentages of foetal haemoglobin. Nature. 1959;184 (Suppl 24): 1877–1878 Journal of Clinical Apheresis DOI 10.1002/jca
6
Ballas and Lyon
16. Steiner RM, Ballas SK, Castro O, Pomerantz K, Embury S, Kutlar A, Burchott S, Bagasra O. Retroviral burden and disease outcome in HIV-I infected patients with sickle cell anemia (SS) (Abstract #305). Presented at National Sickle Cell Disease Conference, Washington DC, 1997. 17. Ballas SK, Cai SP, Gabuzda T, Chehab FF. Molecular basis of asymptomatic beta-thalassemia major in an African American individual. Am J Med Genet 1997;69:196–199. 18. Janes SL, Pocock M, Bishop E, Bevan DH. Automated red cell exchange in sickle cell disease. Br J Haematol 1997;97:256– 258. 19. Eckman JR. Techniques for blood administration in sickle cell patients. Semin Hematol 2001;38 (1 Suppl 1):23–29. 20. Ballas SK. Effect of alpha-globin genotype on the pathophysiology of sickle cell disease. Pediatr Pathol Mol Med 2001;20:107–121.
Journal of Clinical Apheresis DOI 10.1002/jca
21. Harms BA, DeHaas DR Jr., Starling JR. Diagnosis and management of genitofemoral neuralgia. Arch Surg 1984;119:339–341 22. Teixeira MJ, Aun R, de Oliveira Junior JO, Segushi HH, de Almeida GM. Genitofemoral neuralgia. Rev Hosp Clin Fac Med Sao Paulo 1982;37:236–240. 23. Serjeant G. Blood transfusion in sickle cell disease: a cautionary tale. Lancet 2003;361:1659–1660. 24. Ayache S, Herman JH, Hall N, Kent T, Ballas SK. Plasma exchange may benefit acute chest syndrome in sickle cell disease. J Clin Apheresis 2007;22:82–83. 25. Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, McMahon RP, Bonds DR. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the multicenter study of hydroxyurea in sickle cell anemia. N Engl J Med 1995;332:1317–1322.
Safety and Efficacy of Blood Exchange Transfusion for Priapism Complicating Sickle Cell Disease Samir K. Ballas* and David Lyon† Cardeza Foundation for Hematologic Research, Department of Medicine, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania Background: Priapism is unwanted painful penile erection that affects about 36% of boys and men with sickle cell disease (SCD) most of whom have sickle cell anemia. Clinically, priapism could be stuttering, minor, or major. The first two types are mild, last < 4 h, are usually treated at home, have good prognosis with normal sexual function. The major type of priapism lasts >4 h, associated with severe pain, requires hospitalization; often does not respond to medical treatment and may require shunt surgery. Untreated major priapism and surgical intervention often cause impotence. In this study, we report our 15-year experience in treating adult patients with SCD and major priapism with blood exchange transfusion after being refractory to other medical therapies. Methods: Adult male African Americans patients with SCD and major priapism were enrolled in this study and followed for 15 years. A Haemonitics V-50 machine was initially used for whole blood exchange and was later replaced with Cobe Spectra machine for RBC exchange. Results: We used 239 blood exchanges requiring 1,136 RBC units. We maintained a post-exchange hemoglobin level of about 10 g/dL and hemoglobin S level < 30%. None of the patients had any neurological complications such as headache, seizures, neurological deficits, or obtundation post-exchange. Conclusion: Together, the data indicate that blood exchange transfusion for the treatment of patients with SCD C 2015 Wiley Periodicals, Inc. and major priapism is efficacious and safe. J. Clin. Apheresis 00:000–000, 2015. V Key words: impotence; shunt surgery; neurological complications; safety
INTRODUCTION
Priapism is unwanted and sustained painful erection lasting 4 or more hours. It is a common complication of sickle cell disease (SCD) affecting 89% of males with at least one episode of priapism by the time they are 20 years old [1]. It is most common in patients with SS, who account for approximately 80–90% of reported cases [2–5]. However, it does occur in all forms of SCD including Hb SC disease, sickle thalassemia, and in sickle trait [2,6]. Clinically, priapism may be stuttering, minor, or major. Stuttering priapism is the occurrence of short, repetitive, and reversible painful episodes with detumescence occurring within 30% in SCD increases whole blood viscosity significantly compared to normal. From Ballas SK. Sickle Cell Pain, 2nd Ed. Washington, DC: IASP; 2014. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.] TABLE IV.
Parameter Hemoglobin
Hematocrit
Reported Hematologic Values before and after Red Blood Cell Exchange Transfusion Pre-exchange
Post-exchange
(1) 6.3
(1) 13.5
(6) 7.3 6 1.4
(6) 12.1 6 1.1
(166) 7.6 6 1.8 N/A
(162) 9.1 6 1.5a N/A
N/A
N/A
(161) 22.8 6 5.5
(167) 26.8 6 4.8
Reference Siegel et al., 1993 [14] Rackoff et al., 1992 [13] This report Siegel et al., 1993 [14] Rackoff et al., 1992 [13] This report
N/A: not available. a P < 0.001 compared to the values in the first two rows.
viscosity. This dual cause of blood viscosity was reported in a patient with SS and acute chest syndromes (ACS) where blood exchange transfusion was not effective in resolving the ACS despite the reduction of Hb S to < 30% [24]. Plasmapheresis following blood exchange transfusion in this patient was effective in resolving the ACS [24].
1. US Department of Health and Human Services. Expert Panel Report. Evidence-Based Management of Sickle Cell Disease. Bethesda, MD: National Institutes of Health, National Heart, Lung and Blood Institute; 2014. 2. Adeyoju AB, Olujohungbe AB, Morris J, Yardumian A, Bareford D, Akenova A, Akinyanju O, Cinkotai K, O’Reilly PH. Priapism in sickle-cell disease; incidence, risk factors and complications—an international multicentre study. BJU Int 2002;90:898–902. 3. Powars DR, Johnson CS. Priapism. Hematol Oncol Clin North Am 1996;10:1363–1372. 4. Rogers ZR. Priapism in sickle cell disease. Hematol Oncol Clin North Am 2005;19:917–928, viii. 5. Sharpsteen JR Jr., Powars D, Johnson C, Rogers ZR, Williams WD, Posch RJ. Multisystem damage associated with tricorporal priapism in sickle cell disease. Am J Med. 1993;94:289–295. 6. Okpala I, Westerdale N, Jegede T, Cheung B. Etilefrine for the prevention of priapism in adult sickle cell disease. Br J Haematol 2002;118:918–921. 7. Emond AM, Holman R, Hayes RJ, Serjeant GR. Priapism and impotence in homozygous sickle cell disease. Arch Intern Med 1980;140:1434–1437. 8. Serjeant GR, de Ceulaer K, Maude GH. Stilboestrol and stuttering priapism in homozygous sickle-cell disease. Lancet 1985;2: 1274–1276. 9. Mantadakis E, Ewalt DH, Cavender JD, Rogers ZR, Buchanan GR. Outpatient penile aspiration and epinephrine irrigation for young patients with sickle cell anemia and prolonged priapism. Blood 2000;95:78–82. [PMID: 10607688] 10. Baron M, Leiter E. The management of priapism in sickle cell anemia. J Urol 1978;119:610–611. 11. Seeler RA. Intensive transfusion therapy for priapism in boys with sickle cell anemia. J Urol 1973;110:360–363. 12. Talacki CA, Ballas SK. Modified method of exchange transfusion in sickle cell disease. J Clin Apher 1990;5:183–187. [PMID: 2228996] 13. Rackoff WR, Ohene-Frempong K, Month S, Scott JP, Neahring B, Cohen AR. Neurologic events after partial exchange transfusion for priapism in sickle cell disease. J Pediatr 1992;120:882–885 14. Siegel JF, Rich MA, Brock WA. Association of sickle cell disease, priapism, exchange transfusion and neurological events: ASPEN syndrome. J Urol 1993;150(5 Part 1):1480–1482. 15. Betke K, Marti HR, Schlicht I. Estimation of small percentages of foetal haemoglobin. Nature. 1959;184 (Suppl 24): 1877–1878 Journal of Clinical Apheresis DOI 10.1002/jca
6
Ballas and Lyon
16. Steiner RM, Ballas SK, Castro O, Pomerantz K, Embury S, Kutlar A, Burchott S, Bagasra O. Retroviral burden and disease outcome in HIV-I infected patients with sickle cell anemia (SS) (Abstract #305). Presented at National Sickle Cell Disease Conference, Washington DC, 1997. 17. Ballas SK, Cai SP, Gabuzda T, Chehab FF. Molecular basis of asymptomatic beta-thalassemia major in an African American individual. Am J Med Genet 1997;69:196–199. 18. Janes SL, Pocock M, Bishop E, Bevan DH. Automated red cell exchange in sickle cell disease. Br J Haematol 1997;97:256– 258. 19. Eckman JR. Techniques for blood administration in sickle cell patients. Semin Hematol 2001;38 (1 Suppl 1):23–29. 20. Ballas SK. Effect of alpha-globin genotype on the pathophysiology of sickle cell disease. Pediatr Pathol Mol Med 2001;20:107–121.
Journal of Clinical Apheresis DOI 10.1002/jca
21. Harms BA, DeHaas DR Jr., Starling JR. Diagnosis and management of genitofemoral neuralgia. Arch Surg 1984;119:339–341 22. Teixeira MJ, Aun R, de Oliveira Junior JO, Segushi HH, de Almeida GM. Genitofemoral neuralgia. Rev Hosp Clin Fac Med Sao Paulo 1982;37:236–240. 23. Serjeant G. Blood transfusion in sickle cell disease: a cautionary tale. Lancet 2003;361:1659–1660. 24. Ayache S, Herman JH, Hall N, Kent T, Ballas SK. Plasma exchange may benefit acute chest syndrome in sickle cell disease. J Clin Apheresis 2007;22:82–83. 25. Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, McMahon RP, Bonds DR. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the multicenter study of hydroxyurea in sickle cell anemia. N Engl J Med 1995;332:1317–1322.
